CASE REPORT Open Access

Struma ovarii associated with pseudo-Meigs’syndrome and elevated serum CA 125: a casereport and review of the literatureWei Jiang, Xin Lu*, Zhi Ling Zhu, Xi Shi Liu, Cong Jian Xu*

Abstract

The association of pseudo-Meigs’ syndrome, elevation of CA 125 to the struma ovarii is a rare condition. So far onlynine cases have been reported in English literature through MEDLINE search. Here we report a 46-year-old case ofthe struma ovarii, presented with ascites, hydrothorax, right ovarian mass and elevated serum CA 125 level. Thesefindings were misdiagnosed for an ovarian malignancy at the first impression. Immediate resolution of the ascites,hydrothorax and normalization of the serum CA 125 level were followed by ovarian mass removal. Struma ovariicould be a rare cause of ascites, hydrothorax, ovarian mass and elevated CA 125. This rare condition should beconsidered in the differential diagnosis in patents with ascites and pleural effusions but with negative cytology.

BackgroundStruma ovarii is a rare ovarian neoplasm derived fromgerm cells in a mature teratoma. This tumor is generallybenign, although malignant transformation has beenreported [1]. The preoperative diagnosis is generally dif-ficult. Thyroid hormones may be produced and in a fewcases asymptomatic women may develop definitive clini-cal hypothyroidism after resection of struma ovarii. Wehere report an unusual case of a 46-year-old womanpresented with ascites, right ovarian mass, and elevatedCA 125 level, which was suspicious for an ovarianmalignancy and underwent a total hysterectomy andbilateral salpingo-oophorectomy. The pathologic diagno-sis was struma ovarii, a specialized ovarian teratomacomposed predominantly of mature thyroid tissue. Thepostoperative period was uneventful and her thyroidfunction was normal. We had reviewed the related lit-eratures in this report as well.

Case presentationThe present case is a 46-year-old, female, gravida 1, para1, who was admitted to a local hospital, complaining offatigue, anorexia, and abdominal swelling. Her medicalhistory included nothing special. Physical examination

revealed a palpable mass in the lower abdomen. A thor-acoabdominal CT scan showed marked pleural effusionand a heterogeneous mass, large ascites with many nod-osity images in the pelvic wall and considered as malig-nant tumor of ovary.She was then transferred to our hospital for further

treatment in September, 2009. The patient’s serum CA125 level was 1230.9 U/mL, while CEA (2.6 ng/ml), AFP(14.2 ng/ml), CA 199 (14.8 U/ml), and CA 153 (7.8 U/ml)levels were within the normal range. Abdominal ultraso-nography showed a heterogeneous, multiloculated mass,with a moderate amount of ascites, and subsequent trans-vaginal ultrasonography revealed a large complex pelvicmass, 16 cm largest dimension, of probable adnexal originwith low blood resistance flow within the tumor. Theuterus was normal in size. Abdominal paracentesis yielded2 liters of yellow serous fluid consistent with an exudativeprocess. Microscopy and cytology revealed only reactivemesothelial cells without malignant cells.The patient was arranged for an exploratory laparot-

omy. Six liters of straw-colored ascites was evacuated.The uterus was in normal size and the left ovary mea-sured 3 × 2 × 2 cm with a normal appearance. A 20 ×18 × 15 cm complex, multicystic mass, without evidenceof external excrescences, had replaced the right ovary.There was no evidence of intraperitoneal (ie. omenta,the surface of convolutions, appendix, liver, etc) spreadof disease or retroperitoneal adenopathy. And right

* Correspondence: xinludoc@163.com; xucj@hotmail.comDepartment of Gynecology, Obstetrics and Gynecology Hospital, FudanUniversity, Shanghai, P.R. China

Jiang et al. Journal of Ovarian Research 2010, 3:18http://www.ovarianresearch.com/content/3/1/18

© 2010 Jiang et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative CommonsAttribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction inany medium, provided the original work is properly cited.

salpingo-oophorectomy was performed. A frozen sectionof the right ovarian mass was interpreted as strumaovarii. As strongly insisted by the patient and her familymember, a subsequent hysterectomy and left salpingo-oophorectomy were performed according to theinformed consent.Post operative thyroid function test including serum

levels of TT3 (1.78 nmo1/L), TT4 (82.5 nmo1/L), FT3(8.2 pmol/L), FT4 (30.5 pmol/L) and TSH (2.3 mU/ml)were performed on day two, which were within normallimits. The level of CA 125 was decreased to 817 U/mL.The final pathology revealed right struma ovarii withbenign thyroid tissue confined to the ovary (Figure 1).The uterus, left ovary, fallopian tube were histologicallyunremarkable and the cytologic evaluation of the asciticfluid showed no evidence of malignant cells.The patient recovered uneventfully and was dis-

charged home on the ninth postoperative day with a CA125 level of 485 U/mL. Following up three months afterher surgery, she had no evidence of ascites and theserum levels of CA 125 was in normal range, she wassymptomatically much improved from her preoperativecondition and received hormone replacement therapy.

DiscussionMature cystic teratomas account for approximately 20%of all ovarian tumors. Of these, approximately 15% con-tain normal thyroid tissue. Struma ovarii is a monoder-mal variant of ovarian teratoma, which predominantly

contains thyroid tissue (greater than 50%) and was firstdescribed by Von Klden in 1895 and Gottschalk in 1899[2]. It constitutes about 2.7% of ovarian teratomas. It isusually a benign condition although occasionally, malig-nant transformation is observed. Preoperative clinicaldiagnosis of struma ovarii, however, is very difficult.Despite containing thyroid tissue, only 5% of struma

ovarii have features of hyperthyroidism [3]. Ascites hasbeen reported in one-third of cases [2]. However,uncommon is the association of ascites and hydrothoraxwith this tumor [2]. Meigs first described the syndromeconsisting of ovarian fibroma/thecoma, with ascites andhydrothorax, characterized by the resolution of symp-toms with removal of the benign tumor [2]. Meigs’ syn-drome proposed to benign and solid tumors with thegross appearance of a fibroma (fibroma, thecoma, granu-losa cell tumor), accompanied by ascites and hydro-thorax. While similar clinic manifestations presented inother conditions was termed as pseudo-Meigs syn-drome. The ascitic and pleural fluids in Meigs’ andpseudo-Meigs’ syndrome are usually serous, but may beserosanguinous. The origin of the effusions remainsobscure, although some mechanisms have been sug-gested such as active fluid secretion by the tumor orperitoneum, venous and/or lymphatic obstruction, lowserum protein and inflammatory products [4].In the literature, very few reports have been published

on struma ovarii associated to ascites and elevatedCA125 [5-8]. In both cases, patients presented with

Figure 1 Microscopic appearance of the right ovary showing thyroid follicles of varying sizes. (H & E, 100×).

Jiang et al. Journal of Ovarian Research 2010, 3:18http://www.ovarianresearch.com/content/3/1/18

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ascites but without pleural effusions. A MEDLINE searchof the English language literature provides only nine casereport describing struma ovarii presenting as pseudo-Meigs’ syndrome with an elevated CA 125 level can initi-ally suggest ovarian carcinoma [9-16]. (Table 1) Wedescribe an additional case to the tenth reported in theliterature with struma ovarii associated with pseudo-Meigs syndrome and elevated CA 125, which shows ana-logies with the ones reported in the literature. It differs insome important respects. Firstly the patient’s age, this ismuch younger than that when the majority of thesetumors occur i.e. in the fifties. Secondly, the patientunderwent a wide resection operation because of thestrong desire of both the patient and her husband andreceived a hormone replacement therapy subsequently.The elevation of CA 125 may have been secondary to

the presence of ascites; however, its level was muchhigher than that typically seen with ascites of benignorigin. An ovarian mass with ascites and elevated serumCA 125 level in a woman generally suggest a malignancyprocess. So the present case with the clinic findings ofascites, hydrothorax, markedly elevated serum CA 125and a large complex pelvic mass in a woman stronglysuggest pelvic malignancy before operation. But com-plete remission of the ascites, hydrothorax, and CA125was obtained after surgery without any adjuvant therapy.

ConclusionThis report emphasizes that there are benign gynecolo-gical conditions might show clinical, ultrasonographic

and biochemical signs suggestive of malignancy. Theyrarely should be considered as the benign diseases inthe differential diagnosis when the patients presentedwith ascites, elevated serum CA 125 and pleural effu-sions, but with negative cytologic examination.

List of abbreviationsCT: computed tomography; TSH: thyroid stimulating hormone; CEA:carcinoembryonic antigen; AFP: alpha-fetoprotein; T3: triiodothyronine; T4:thyroxine; TT3: total T3; TT4: total T4; FT3: free T3; FT4: free T4.

Competing interestsThe authors declare that they have no competing interests.

Authors’ contributionsWJ drafted the manuscript. XL, ZJZ, CJX, XSL are involved in design,acquisition, interpretation and manuscript preparation. All authors had readand approved the final manuscript.

Authors’ informationWJ, XL, ZLZ, CJX, XSL: Department of Gynecology, Obstetrics andGynecology Hospital, Fudan University, Shanghai, P. R. China.

ConsentWritten informed consent was obtained from the patient for publication ofthis case report and any accompanying images. A copy of the writtenconsent is available for review by the Editor-in-Chief of this journal.

AcknowledgementsWe thank Dr. Xianrong Zhou at pathology department of our hospital for hiskindly analysis of patient’s tissue sample.

Received: 18 April 2010 Accepted: 29 July 2010 Published: 29 July 2010

References1. McCluggage WG, Bissonnette JP, Young RH: Primary malignant melanoma

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Table 1 Struma ovarii associated with Pseudo-Meigs’ syndrome and elevated CA125 level: reported cases

Author No. ofpatients

Age(years)

Clinical symptoms CA125(U/mL)

Treatments Prognosis &follow up time

Bethune Met al. (9)

1 62 Acute hydrothoraces, dyspneaand abdominal swelling

1570 Total hysterectomy and bilateral Salpingo-oophorectomy

Well, 5 months

Long CYet a.l (10)

2 5378

Both with abdominal swelling,pain, or dyspnea

233335

Both with total hysterectomy and bilateral Salpingo-oophorectomy

Well, 10 monthsWell, 6 months

Huh JJet al. (11)

1 65 Abdominal distension,dyspnea

402 Total hysterectomy and bilateral Salpingo-oophorectomy and appendectomy and omental biopsy

Well, 4 months

Loizzi Vet al. (12)

1 65 Dyspnea,diffuse abdominal pain

161 Not included Well, 2 months

Mitrou Set al.(13)

1 58 Large pelvic mass, ascites 1028 Total hysterectomy and bilateral Salpingo-oophorectomy

Well, 12 months

Paladini D,et al(14)

1 42 Ascites, fever, diarrhea,vomiting and significantweightloss.

2548 Right Salpingo-oophorectomy Well, 6 months

ObeidatBR, et al(15)

1 67 Dyspnea, abdominal swelling,pelvic mass

176 Total hysterectomy and bilateral Salpingo-oophorectomy

Well, 6 months

Rana V,et al(16)

1 70 Progressive ascites, bilateralpleural effusion

284 total abdominal hysterectomy with bilateral Salphingo-opherectomy and partial omentectomy

Well, 3 months

Presentcase

1 46 Abdominal swelling,fatigue, weight loss

1230.9 Total hysterectomy and bilateral Salpingo-oophorectomy

Well, 3 months

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14. Paladini D, Vassallo M, Sglavo G, Nappi C: Struma ovarii associated withhyperthyroidism, elevated CA 125 and pseudo-Meigs syndrome maymimic advanced ovarian cancer. Ultrasound Obstet Gynecol 2008,32(2):237-238.

15. Obeidat BR, Amarin ZO: Struma ovarii with pseudo-Meigs’syndrome andelevated CA125 levels. J Obstet Gynaecol 2007, 27(1):97-98.

16. Rana V, Srinivas V, Bandyopadhyay S, Ghosh SK, Singh Y: Bilateral benignnon functional struma ovarii with Pseudo-Meigs’ syndrome. Indian JPathol Microbiol 2009, 52(1):94-96.

doi:10.1186/1757-2215-3-18Cite this article as: Jiang et al.: Struma ovarii associated with pseudo-Meigs’ syndrome and elevated serum CA 125: a case report and reviewof the literature. Journal of Ovarian Research 2010 3:18.

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